Hirschsprung disease

(redirected from congenital aganglionic megacolon)

con·gen·i·tal meg·a·co·lon

, megacolon congenitum
congenital dilation and hypertrophy of the colon due to absence (aganglionosis) or marked reduction (hypoganglionosis) in the number of ganglion cells of the myenteric plexus of the rectum and a varying but continuous length of gut above the rectum; seen in humans and dogs.
Farlex Partner Medical Dictionary © Farlex 2012

Hirschsprung disease

A condition of infant onset characterised by the absence of myenteric nerves in the distal colon, extending proximally from the anus for a variable distance. It affects an estimated 1:5000 live births, and is more common in males (male:female ratio, 4:1); up to 10% have Down syndrome. 

Clinical findings
Failure to pass meconium or stool in first 24 hours post-partum; abdominal distention; vomiting; constipation at birth. Peristalsis is absent in aganglionic segment, causing proximal pseudo-obstruction and gross dilatation, enterocolitis and perforation. In older children, Hirschsprung disease is characterised by chronic constipation, abdominal distention and stunted growth.

Diagnosis
By rectal suction biopsy; must include submucosa, taken at least 2 cm above pectinate line.

Management
Resection of aganglionic segment, guided by intraoperative frozen sections from myenteric plexus.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.

con·gen·i·tal meg·a·co·lon

, megacolon congenitum (kŏn-jen'i-tăl meg'ă-kō-lŏn, kon-jen'i-tŭm)
Congenital dilation and hypertrophy of the colon due to absence (aganglionosis) or marked reduction (hypoganglionosis) in the number of ganglion cells of the myenteric plexus of the rectum and a varying but continuous length of colon above the rectum.
Synonym(s): Hirschsprung disease.
Medical Dictionary for the Health Professions and Nursing © Farlex 2012

Hirschsprung disease

Hirschsprung disease is a congenital abnormality (birth defect) of the bowel in which there is absence of the ganglia (nerves) in the wall of the bowel. Nerves are missing starting at the anus and extending a variable distance up the bowel. This results in megacolon (massive enlargement of the bowel) above the point where the nerves are missing. (The nerves are needed to assist in the natural movement of the muscles in the lining of the bowels that move bowel contents through.)
Mentioned in: Ostomy
Gale Encyclopedia of Medicine. Copyright 2008 The Gale Group, Inc. All rights reserved.

Hirschsprung,

Harald, Danish physician, 1830-1916.
Hirschsprung disease - congenital dilation and hypertrophy of the colon. Synonym(s): congenital megacolon
Medical Eponyms © Farlex 2012
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